Aparna Ramasubramanian - Ocular Oncology

Management of retinoblastoma is complex as well as highly customized for every single child. The choice of treatment depends on many factors including age of the patient at presentation, tumor laterality, tumor size and location, macular involvement, vitreous and/or subretinal seeding, tumor relationship to surrounding tissues (optic disc, choroid, iris, sclera, and orbit), tumor staging, risk for metastasis, and visual potential []. It involves certain considerations such as systemic status, overall prognosis as well as family desires, social perception, and cost-effectiveness of treatment in a particular financial setting.

The principal objective of retinoblastoma treatment is child survival, followed by globe salvage and preservation of vision. It is a challenge to cure the disease and preserve the globe while maximizing visual potential and minimizing toxicity. A multidisciplinary approach at a tertiary care center is fundamental in the treatment of retinoblastoma with an organized team comprising ocular oncologist, pediatric oncologist, ocular pathologist, pediatrician, interventional radiologist, radiotherapist, and genetic counselor [].

Various modalities in the treatment of retinoblastoma can be classified into focal therapy (transpupillary thermotherapy, laser photocoagulation, cryotherapy, plaque brachytherapy), local therapy (intra-arterial chemotherapy, enucleation, external beam radiotherapy/EBRT), and systemic therapy (intravenous chemotherapy). Small tumors are primarily treated with focal therapy, while local and systemic modalities are used in the treatment of advanced retinoblastoma [].

There has been remarkable advancement in the conservative treatment of intraocular as well as orbital retinoblastoma over the last decade, using different routes of drug delivery, new chemotherapeutic agents, and novel radiation treatment modalities in order to improve success rates and minimize the adverse effects of conventional treatment.

Conservative treatment modalities which salvage the globe as well as preserve functional vision are increasingly being used for less advanced disease (groups AD ICIOR) []. These include systemic chemotherapy, intra-arterial chemotherapy, focal consolidation, radiation treatment with plaque brachytherapy, and local injections of chemotherapeutic agents through the intravitreal/subtenons route in addition to systemic chemotherapy.